Adrenal cortical tumors in childhood

In a 41-year period, 18 children with a diagnosis of an adrenal cortical tumor were identified (14 carcinoma: 4 adenoma). The majority of patients had clinical signs of endocrine dysfunction at presentation, with virilization (11 patients) and a cushingoid appearance (8 patients) the commonest findings. Abnormal biochemical activity was identified in 16 tumors (94%). The primary treatment in 17 patients was surgical. In addition, 12 children, all with carcinomas, had radiotherapy. Of those children with a carcinoma, 12 are dead, with a median survival of 52 months (range 1-317 months). The three second primary tumors all developed at sites within the field of previous radiotherapy; and proved fatal at 127, 176, and 317 months (median 207 months). This series confirms the poor prognosis in adrenocortical carcinoma in childhood, but a complete resection is compatible with cure of the primary disease. The frequency of second, fatal, primary tumors is of particular concern and long-term follow-up is mandatory in survivors, especially if radiotherapy was part of the treatment protocol.